Prolonged duration of post-traumatic amnesia: A sensitive classification for predicting cognitive outcomes in children recovering from traumatic brain injury.

OBJECTIVE: A paucity of data exists regarding the duration of post-traumatic amnesia (PTA) as a predictor of cognitive functioning among children after traumatic brain injury (TBI). The study aimed to assess the relationship between PTA duration and areas of neurocognitive function among the pediatric population in the sub-acute phase of recovery and rehabilitation. METHODS: Data were collected from medical files on 103 children aged 5.5-16.5 hospitalized at a pediatric rehabilitation department with a diagnosis of moderate-severe TBI (msTBI) between the years 2004-2019. The Children Orientation and Amnesia Test was used to evaluate PTA duration. Measures of high-order cognitive abilities of attention and executive function were collected using the Test of Everyday Attention-Child version (TEA-Ch). RESULTS: Three PTA duration groups were assembled out of a cluster analysis: "Long PTA" (M = 21 days), "Very Long PTA" (M = 47 days), and "Extremely Long PTA" (M = 94 days). Analyses revealed that the "Long PTA" group preformed significantly better than the "Very Long PTA" and "Extremely Long PTA" groups on all TEA-Ch measures, that is, Selective Attention, Attentional Control Switching, and Sustained Attention. CONCLUSIONS: This study is the first to demonstrate that PTA duration is a useful predictor of high-order cognitive functions among children with msTBI in the sub-acute phase of recovery and rehabilitation. The findings emphasize the importance of using a more sensitive classification of prolonged PTA durations to improve outcome prediction and allocation of resources to those who can benefit most after severe brain injuries.

Growing up with Bilateral parieto-occipital injury: over ten years of clinical observation and neuropsychological follow-up.

This paper presents a follow-up of a child with Balint's syndrome over more than a decade. The patient experienced traumatic brain injury before age 12, resulting in bilateral occipito-parietal infarctions and a clinical presentation of Balint's syndrome. Neuropsychological assessments at three time points showed average verbal abilities alongside persistent difficulties in visual orientation, mirrored in the patient's daily life. Her outstanding compensatory abilities in the face of these impairments are discussed with respect to the recruitment of the ventral visual stream and the role of top-down processing. This profile may help to determine interventions for younger patients with similar lesions.

Mitochondrial augmentation of hematopoietic stem cells in children with single large-scale mitochondrial DNA deletion syndromes.

Patients with single large-scale mitochondrial DNA (mtDNA) deletion syndromes (SLSMDs) usually present with multisystemic disease, either as Pearson syndrome in early childhood or as Kearns-Sayre syndrome later in life. No disease-modifying therapies exist for SLSMDs. We have developed a method to enrich hematopoietic cells with exogenous mitochondria, and we treated six patients with SLSMDs through a compassionate use program. Autologous CD34+ hematopoietic cells were augmented with maternally derived healthy mitochondria, a technology termed mitochondrial augmentation therapy (MAT). All patients had substantial multisystemic disease involvement at baseline, including neurologic, endocrine, or renal impairment. We first assessed safety, finding that the procedure was well tolerated and that all study-related severe adverse events were either leukapheresis-related or related to the baseline disorder. After MAT, heteroplasmy decreased in the peripheral blood in four of the six patients. An increase in mtDNA content of peripheral blood cells was measured in all six patients 6 to 12 months after MAT as compared baseline. We noted some clinical improvement in aerobic function, measured in patients 2 and 3 by sit-to-stand or 6-min walk testing, and an increase in the body weight of five of the six patients suffering from very low body weight before treatment. Quality-of-life measurements as per caregiver assessment and physical examination showed improvement in some parameters. Together, this work lays the ground for clinical trials of MAT for the treatment of patients with mtDNA disorders.

Assessing cognitive functioning in females with Rett syndrome by eye-tracking methodology.

BACKGROUND: While many individuals with severe developmental impairments learn to communicate with augmentative and alternative communication (AAC) devices, a significant number of individuals show major difficulties in the effective use of AAC. Recent technological innovations, i.e., eye-tracking technology (ETT), aim to improve the transparency of communication and may also enable a more valid cognitive assessment. OBJECTIVES: To investigate whether ETT in forced-choice tasks can enable children with very severe motor and speech impairments to respond consistently, allowing a more reliable evaluation of their language comprehension. METHODS: Participants were 17 girls with Rett syndrome (M = 6:06 years). Their ability to respond by eye gaze was first practiced with computer games using ETT. Afterwards, their receptive vocabulary was assessed using the Peabody Picture Vocabulary Test-4 (PPVT-4). Target words were orally presented and participants responded by focusing their eyes on the preferred picture. RESULTS: Remarkable differences between the participants in receptive vocabulary were demonstrated using ETT. The verbal comprehension abilities of 32% of the participants ranged from low-average to mild cognitive impairment, and the other 68% of the participants showed moderate to severe impairment. Young age at the time of assessment was positively correlated with higher receptive vocabulary. CONCLUSIONS: The use of ETT seems to make the communicational signals of children with severe motor and communication impairments more easily understood. Early practice of ETT may improve the quality of communication and enable more reliable conclusions in learning and assessment sessions.

The Role of Maternal Distress in the Report of Behavioral and Emotional Problems among Children with Chronic Disabilities.

BACKGROUND: Assessments of psychological symptoms in children often rely on caregivers' (usually mothers') reports. However, the reliability may be affected by the caregivers' own emotional distress (ED). The main objectives of this study were to assess the variability in ED of mothers of children with chronic physical disabilities, and its association with the ratings of their children's emotional and behavioral problems. METHODS: Medical data of children diagnosed with chronic disabilities were analyzed (N = 72). Mothers completed the 12-item General Health Questionnaire (12-GHQ) to measure ED and the Child Behavior Checklist (CBCL) to assess children's emotional and behavioral problems Mothers' ED scores were compared with communitybased counterparts with similar socio-demographic characteristics (N = 657) from the Israel National Health Survey (INHS). RESULTS: Mothers of children with chronic physical disabilities had higher levels of ED compared to mothers in the general population. About 20% of the sample mothers had 12-GHQ scores compatible with DSM- IV depression or anxiety disorders. No differences in ED were found according to the type of child's disability or IQ score. Marked differences in CBCL scores were reported by mothers with high versus low ED, controlling for baseline maternal and child characteristics. CONCLUSIONS: High levels of maternal ED were associated with mothers' reports on child's behavioral and emotional problems.This may contaminate the reliability of parental reports on their child's psychological state.

The effect of age-at-testing on verbal memory among children following severe traumatic brain injury.

Memory deficits are a common sequelae following childhood traumatic brain injury (TBI), which often have serious implications on age-related academic skills. The current study examined verbal memory performance using the Rey Auditory Verbal Learning Test (RAVLT) in a pediatric TBI sample. Verbal memory abilities as well as the effect of age at-testing on performance were examined. A sample of 67 children following severe TBI (age average = 12.3 ± 2.74) and 67 matched controls were evaluated using the RAVLT. Age effect at assessment was examined using two age groups: above and below 12 years of age during evaluation. Differences between groups were examined via the 9 RAVLT learning trials and the 7 composite scores conducted out of them. Children following TBI recalled significantly less words than controls on all RAVLT trials and had significantly lower scores on all composite scores. However, all of these scores fell within the low average range. Further analysis revealed significantly lower than average performance among the older children (above 12 years), while scores of the younger children following TBI fell within average limits. To conclude, verbal memory deficits among children following severe TBI demonstrate an age-at-testing effect with more prominent problems occurring above 12 years at the time of evaluation. Yet, age-appropriate performance among children below 12 years of age may not accurately describe memory abilities at younger ages following TBI. It is therefore recommended that clinicians address child's age at testing and avoid using a single test as an indicator of verbal memory functioning post TBI.